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angioendotheliomatosis
Etiology:
- malignant phenotype most common
- benign reactive variant (reactive angioendotheliomatosis is a rare, poorly defined disorder
- etiololgy depends on offending disease
Epidemiology:
- 100 cases of malignant angioendotheliomatosis & ~40 cases reactive angioendotheliomatosis have been described worldwide
Pathology:
- proliferation of histiocytes within vascular lumina
- sludging effect of circulating malignant lymphoid cells
- secondary intravascular thrombi & obliteration of the vessels
Laboratory:
- complete blood count
- anemia
- thrombocytopenia
- leukocyte count variable
- peripheral blood smear
- serum chemistries
- serum LDH elevated
- serum AST & serum ALT elevated
- serum alkaline phosphatase elevated
- cryofibrinogen in plasma may be elevated
Management:
- treat as B-cell lymphoma
- prognosis
- mortality 80% within 1 year (malignant form)
- reactive angioendotheliomatosis has a good prognosis -regression common when underlying disease, if discovered, is successfully treated
Notes:
- classification of diffuse large B-cell lymphoma derived from Snomed (otherwise would be classified as B-cell lymphoma)
General
diffuse large B-cell lymphoma (DLBCL)
References
- Schwartz RA, Elston DM
Medscape: Angioendotheliomatosis.
http://emedicine.medscape.com/article/1085936-overview